Shone's syndrome (SS) is a rare congenital heart disease (CHD) characterised by four defects: supravalve mitral membrane, parachute mitral valve, subaortic stenosis and aortic coarctation. Incomplete forms of SS are more common and consist in the coexistence of only two or three of these left heart obstructive anomalies. To date there is limited data on prenatal diagnosis of this CHD. We report an incomplete SS diagnosed prenatally and discuss its parental counselling. A 44-year-old, G3P2, healthy woman was referred to our Fetal Medicine Department at 21 weeks of gestation with ultrasound features of CHD. There was no family history of cardiac defects and the first trimester screening for chromosomal abnormalities and major defects was normal. Fetal echocardiography identified mitral and aortic valves stenosis, with turbulent anterograde flow, subaortic interventricular communication with bidirectional flow, hypoplastic ascending aorta and aortic arch with retrograde flow to its distal portion, dilatation of right atrial and ventricle and hypoplastic left ventricle. No extracardiac anomalies were observed. There were no abnormal findings in karyotype and microarray analysis of the amniotic fluid. After a multidisciplinary evaluation, parents were informed about the clinical prognosis and opted for termination of pregnancy. Anatomopathological study corroborated the ultrasound findings. A congenital mitral valve obstruction is the main pathological event that leads to the maldevelopment of left heart outflow tract. The degree of this underdevelopment will influence clinical presentation at birth, need for medical/surgical intervention and morbimortality. In this case, prenatal echocardiography revealed features compatible with incomplete SS that was progressing to a hypoplastic left heart syndrome. These findings were crucial to provide parents the proper counseling, including implications underlying this pathology and therapeutic options.
Introduction: A case of acute myopericarditis related to radiotherapy is presented. Incidence is difficult to affirm but chronic presentation is much more common. Diagnosis is challenging and a high suspicion level is important to establish it.
Introducao: Sendo o Canal Arterial a anomalia cardiovascular mais frequente no periodo neonatal, sobretudo em recem-nascidos pre-termo, os autores conduziram um estudo sobre esse tema numa unidade de cuidados intensivos neonatais. Objectivos: Avaliar as consequencias para o recem-nascido de muito baixo peso do diagnostico precoce ou tardio da patencia de canal arterial. Metodos: Estudo retrospectivo incidindo sobre 46 recem-nascidos de peso inferior a 1500g, internados na Unidade de Cuidados Intensivos a Recem-Nascidos da Maternidade Dr. Alfredo da Costa, durante o periodo de Janeiro de 1994 a Junho de 1996, e que tiveram canal arterial sintomatico isolado. Foram analisados o tempo total de ventilacao, oxigenioterapia, uso previo de surfactante, associacao com displasia broncopulmonar, hemorragia intraventricular, retinopatia da prematuridade, enterocolite necrosante e numero de ciclos de indometacina, de acordo com a data de diagnostico da patencia de canal arterial. Resultados: O diagnostico de patencia de canal arterial foi feito ate ao 5.° dia em 25 recem-nascidos (grupo precoce) e apos o 5.° dia em 21 (grupo tardio). Oitenta e quatro porcento dos recem-nascidos do grupo precoce e 62% do grupo tardio, fizeram surfactante. Foram ventiladas 44 criancas. A eficacia da indometacina foi de 95%. Em 2 houve necessidade de tratamento cirurgico. A displasia broncopulmonar ocorreu em 40% no grupo precoce e em 25% no grupo tardio; a retinopatia da prematuridade em 60% versus 45%; a enterocolite necrosante em 16% versus 14% e a hemorragia intraventricular em 48% versus 35%. Conclusao: Apesar das aparentes diferencas encontradas nao foi possivel separar como duas entidades estatisticamente distintas a patencia de canal arterial diagnosticada precoce ou tardiamente, quer em termos de factores causais, quer em termos de consequencias. A administracao de surfactante e a morbilidade estiveram relacionadas com uma menor idade gestacional e com a apresentacao clinica mais precoce, mas so se encontrou uma associacao estatisticamente significativa entre a utilizacao de surfactante e a idade de diagnostico.
We report a surgical series of submitral aneurysm in children.Between March 2011 and December 2015, eight consecutive patients less than 18 years old with submitral aneurysm underwent surgical correction.Six patients were female, the mean age was 7 ± 3.8 years old, and mean weight was 21.4 kg. Six patients were in NYHA functional class III or IV. Six patients underwent repair via a transatrial approach, another with a transatrial combined with transaneurysmal approach, and another with a transventricular approach. There were no in-hospital deaths but one 30-day mortality. One patient required reoperation. Two patients required mitral valve replacement. At discharge, one patient had severe and another had moderate mitral regurgitation. The mean follow-up time was 26.4 months and five patients were alive. No reintervention was required.Submitral aneurysm is not restricted to adults. Heart failure is the commonest clinical presentation in the pediatric age. The transatrial approach is feasible, safe, and associated with good short-term results. The mitral valve can be preserved in the majority of cases.
Cancer patients appear to be a vulnerable group in the COVID-19 pandemic. This study aims to compare clinical characteristics and outcomes of cancer and non-cancer patients with COVID-19 admitted to the ICU. All COVID-19 cancer patients (cases) admitted to a Portuguese ICU between March 2020 and January 2021 were included and matched on age, sex and comorbidities with COVID-19 non-cancer patients (controls); 29 cases and 29 controls were enrolled. Initial symptoms were similar between the two groups. Anemia was significantly superior among cases (76% vs. 45%;
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